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2006-04-04-15 Neuroblastoma © Cuillier www.thefetus.net/


Neuroblastoma

Cuillier F, MD*, Carasset G, MD**, Lemaire P***, Deshayes M***

* Department of Gynecology ** Gynecologist, Saint Clothil clinic *** Sonogapher, Moufia’street , Hôpital Félix Guyon - Ile de la Réunion, France

Definition: Neuroblastoma is a sarcoma consisting of malignant neuroblasts, usually arising from cells of the autonomic nervous system (1/3) or the adrenal medulla (2/3). Neuroblastoma is the most common malignant tumor in infancy and early childhood (1).

Approximately:

  • 70% develop in the abdomen, with about half being in the adrenal gland
  • 20% occur in the thoracic cavity
  • 10% occur in all the areas where cells of the neural crest can be found.

The most frequent metastases are in the liver, bones, marrow and skeleton.
We report one case of fetal adrenal mass detected by routine prenatal ultrasound.

Synonyms: Neuroblastoma in situ ; Adrenoblastoma.

Case report: A 35-year-old primigravid woman had a normal 13 and 22 weeks scan. The nuchal translucency and the triple test were strictly normal. At 34 weeks, the patient underwent a routine ultrasound examination. The scan revealed a heterogeneous, hypoechoic and hyperechoic mass, between the upper pole of the right kidney and the diaphragm, measuring 29 x 30 x 32 mm (Figure N°1-2-3-4). The color Doppler demonstrated that the tumor was arising from the adrenal gland and there was a poor vascularization around the tumor (Figure N°5). Fetal biometry, biophysical profile and Doppler examination were normal (Figure N°6). At 34 weeks, a MRI was performed and confirmed the right heterogeneous mass (Figure N°9-10). A adrenal hemorrhage and a neuroblastoma were suspected. The mother did not demonstrate any adrenergic symptomatology or hypertension. Fetal biometry and morphology were otherwise normal. At 35, 37 and 39 weeks, another scans were performed (Figure N°7-8).

At 40 weeks, the baby was delivered (2800g) vaginally.  The Apgar score was 10/10 at 1/5 minutes, respectively. A small right flank mass was palpable on physical examination. Vanilmandelic acid and catecholamine levels were negative in a 24-hour-urine sample. MRI and ultrasound scans confirmed a right solid adrenal mass. Perfusion scan showed contrast enhancement and calcifications suggestive of neuroblastoma (Figure N°11-12).

Preoperative diagnostic evaluation was otherwise normal and included bone marrow aspirate, bone scan, urinary catecholamines and liver function tests. Concentrations of catecholamines metabolites and vanilmandelic acid were not elevated in the neonatal blood. Adrenal scintigraphy was done and confirmed the neuroblastoma diagnosis. A right tumorectomy was performed on day-25.  Pathological examination showed an encapsulated stage I neuroblastoma with favorable histology. No further treatment was performed. After one month, the baby is healthy.

Figure 1,2,3,4 :Transverse view at 34 weeks showing a hypoechoic suprarenal mass